Cerebral thrombotic microangiopathy and antiphospholipid antibodies in Aicardi-Goutieres syndrome--report of two sisters.

Abstract

Cerebral thrombotic microangiopathy was found at autopsy in one of two sisters with Aicardi-Goutieres syndrome, whereas the other revealed increased serum levels of anticardiolipin IgG antibodies (measured only in the living sister); both typical features of systemic lupus erythematosus. These findings add support to the suggestion that Aicardi-Goutieres syndrome and systemic lupus erythematosus are closely related disorders in which dysregulated production of interferon-alpha might play a crucial role.

• Authors: M Rasmussen, K Skullerud, S J Bakke, P Lebon, F L Jahnsen
• Journal: Neuropediatrics (Neuropediatrics) Vol. 36 Issue 1 Pg. 40-4 (Feb 2005) ISSN: 0174-304X [Print] Germany
• PMID: 15776321 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antibodies, Antiphospholipid

Topics

• Antibodies, Antiphospholipid (blood)
• Antiphospholipid Syndrome (immunology, physiopathology)
• Atrophy (immunology, pathology, physiopathology)
• Basal Ganglia Diseases (complications, immunology, pathology, radiotherapy)
• Brain (pathology)
• Child, Preschool
• Female
• Humans
• Infant
• Intracranial Thrombosis (complications, immunology, pathology)
• Lupus Erythematosus, Systemic (complications, diagnostic imaging, immunology)
• Radiography (methods)